Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy

Akihiro Yoshida; Satoru Hagiwara; Tomohiro Watanabe; Naoshi Nishida; Hiroshi Ida; Toshiharu Sakurai; Yoriaki Komeda; Kentaro Yamao; Mamoru Takenaka; Eisuke Enoki; Masatomo Kimura; Masako Miyake; Akira Kawada; Masatoshi Kudo

doi:10.2169/internalmedicine.0673-17

CASE REPORTS

Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy

Akihiro Yoshida, Satoru Hagiwara, Tomohiro Watanabe, Naoshi Nishida, Hiroshi Ida, Toshiharu Sakurai, Yoriaki Komeda, Kentaro Yamao, Mamoru Takenaka, Eisuke Enoki, Masatomo Kimura, Masako Miyake, Akira Kawada, Masatoshi Kudo

Author information

Akihiro Yoshida
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Satoru Hagiwara
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Tomohiro Watanabe
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Naoshi Nishida
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Hiroshi Ida
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Toshiharu Sakurai
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Yoriaki Komeda
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Kentaro Yamao
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Mamoru Takenaka
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan
Eisuke Enoki
Department of Pathology, Kindai University Faculty of Medicine, Japan
Masatomo Kimura
Department of Pathology, Kindai University Faculty of Medicine, Japan
Masako Miyake
Department of Dermatology, Kindai University Faculty of Medicine, Japan
Akira Kawada
Department of Dermatology, Kindai University Faculty of Medicine, Japan
Masatoshi Kudo
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan

Keywords: erythrocyte protoporphyrin, plasma exchange, phlebotomy

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 17 Pages 2505-2509

DOI https://doi.org/10.2169/internalmedicine.0673-17

Details

Abstract

A 27-year-old man bearing an erythropoietic protoporphyria (EPP)-associated ferrochelatase (FECH) mutation was admitted to our hospital for general malaise and marked elevation of the serum levels of hepatobiliary enzymes and bilirubin. Initial treatment with plasma exchange did not reduce the blood protoporphyrin or serum liver enzyme levels, so phlebotomy was started. Surprisingly, weekly phlebotomy normalized the serum levels of liver enzymes, accompanied by a marked reduction in the blood protoporphyrin levels. The clinical course of this case strongly suggests that phlebotomy may be a suitable treatment option for EPP-related hepatopathy.

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